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KMID : 0376119790060010029
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Medical Journal of the Red Cross Hospital
1979 Volume.6 No. 1 p.29 ~ p.35
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송영명
이경숙/신미자/강석철/지제근
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Abstract
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Acute Werdnig-Hoffmann disease is genetically determined progressive degenerative disease of anterior spinal neuron, characterized by fasciculation’s of the tongue usually occurring along with flaccid paralysis of extremities with intact mentality.
We experienced a 5 months old female infant, whose sister died of pneumonia at the age of 2 months, had same symptoms, and signs; revealing hypo tonic state with flaccid extremities only responding to painful stimuli, and paradoxical respiration.
She was alert in mental state and showing typical tongue fasciculation even in quiet state. Muscle biopsy finding was compatible with acute Werdnig-Hoffmann disease and serum enzymes were normal value.
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KEYWORD
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